Marrow Fibrosis in Myelodysplastic Syndromes - Its Significance in the Context of the WHO Classification of Disease and the International Prognostic Scoring System.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1446-1446
Author(s):  
Guntram Buesche ◽  
Arnold Ganser ◽  
Ludwig Wilkens ◽  
Brigitte Schlegelberger ◽  
Hartmut Hecker ◽  
...  
Keyword(s):  
Survival Time ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Who Classification ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Bone Marrow Biopsies ◽  
Health Organization ◽  
Marrow Fibrosis

Abstract Marrow fibrosis (MF) is rarely considered in myelodysplastic syndromes (MDS) although the frequency of this complication ranges from 10 to 50 % in the few reports on this issue, and there are no data on occurrence and significance of this complication in the context of the International Prognostic Scoring System (IPSS) and the World Health Organization (WHO) classification of disease. In a retrospective study, diagnostic bone marrow biopsies from a total of 936 patients with MDS were examined for MF and its relevance to the course of disease. Frequency of MF varied markedly between different types of MDS ranging from 3 % (RARS) to 37 % (MDS, therapy-related; WHO classification, P < 0.000005). Risk of MF furthermore correlated with multilineage dysplasia (P < 0.000005). However, there was no obvious correlation to the IPSS or to karyotype abnormalities. The survival time of patients was significantly reduced by about 50 % from 11 (RAEB-1/-2) - 55 (RARS, RCMD-RS) down to 6 (RAEB-1/-2) - 33 months (RARS, RCMD-RS) in median when MF was detected independently of the IPSS and the classification of disease (FAB, WHO; P = 0.0001). We conclude that MF is an unfavorable complication of MDS significantly shortening the survival time of patients independently of the IPSS and the classification of disease.

Incidence and Clinical Significance of Scoring System in Myelodysplastic Syndromes Including Hematological and Cytogenetic Profiles from a Single Institution

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5102-5102
Author(s):  
Myungshin Kim ◽  
Jihyang Lim ◽  
Yonggoo Kim ◽  
Kyungja Han ◽  
Yoo-Jin Kim ◽  
...  
Keyword(s):  
Scoring System ◽  
Who Classification ◽  
Chromosomal Abnormalities ◽  
Chromosome Analysis ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Good Prediction ◽  
Trisomy 8 ◽  
Long Time ◽  
Health Organization

Abstract Background: Mydelodysplastic syndromes (MDS) are clonal stem cell disorders characterized by hypercellular marrow with ineffective hematopoiesis determining peripheral cytopenia. Recently, the WHO developed a new classification that identifies the following subtypes: RA, RARS, 5q- syndrome (MDS 5q-), RCMD, RCMD with ringed sideroblasts (RCMDS), RAEB-1, RAEB-2 and MDS unclassifiable (MDS-U). The goals of our study were to establish the incidence of chromosome abnormalities in MDS patients, to correlate chromosomal defects with WHO subtype and peculiar clinical-biological findings. Patients and Methods: We studied 242 consecutive MDS patients who were admitted in the hematology department of St. Mary’s hospital from 2000 to 2007. The diagnosis was made according to the World Health Organization classification system. The chromosome analysis of the BM cells at diagnosis was performed by the G-staining method with trypsin and karyotyped according to the ISCN 2005. Results: The median age of patients was 50 (2–84). WHO classification was as follows: RA (n=21, 8.7%), RARS (n=7, 2.9%), RCMD (n=62, 25.6%), RCMDS (n=6, 2.5%), RAEB-1 (n=63, 26.0%), RAEB-2 (n=74, 30.6%), MDSU (n=7, 2.9%), MDS 5q- (n=2, 0.8%). Chromosomal abnormalities were found in 51.2% of patients. The cases with good, intermediate and poor cytogenetic risk features were observed in 55.0%, 29.3%, and 15.7%. Trisomy 8 was most common abnormality in all cases. 1q+, 5q-, 20q- were followed. By International Prognostic Scoring System (IPSS) 13 (5.4%) patients were classified in low group; 141 (58.3%) intermediate-1, 60 (24.8%) intermediate-2, and 28 (11.6%) high were classified. By WHO classification-based prognostic scoring system (WPSS), 14 (5.8%) patients were classified in very low group; 25 (10.3%) low, 57 (23.6%) intermediate, 110 (45.5%) high, and 36 (14.9%) very high were classified. Thirty-six patients (14.9%) progressed into AML. Both IPSS and WPSS was shown to predict survival and leukemia progression, even in a short-term prospective study (P&lt;0.001). Conclusions: It is important to diagnose MDS accurately on the base of cytopenia, bone marrow morphology and clonal chromosomal abnormalities. IPSS and WPSS provide good prediction of survival and risk of leukemic evolution in MDS patients. The evaluation of clinical, hematological and cytogenetic analysis in more cases for long time follow-up will be helpful to standardize diagnosis of MDS.

The changing classification of myelodysplastic syndromes: what’s in a name?

Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 645-655 ◽  
Author(s):  
David P. Steensma
Keyword(s):  
Natural History ◽  
Myelodysplastic Syndromes ◽  
Bone Marrow Failure ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Current Standard ◽  
World Health Organization Classification ◽  
History Of ◽  
Health Organization

AbstractWhat is the most sensible way of organizing the disorderly spectrum of acquired marrow failure states collectively known as the myelodysplastic syndromes (MDS)? While the 2008 version of the World Health Organization classification is the current standard, the recent proliferation of MDS prognostic tools illustrates the usefulness of supplemental information for clinical purposes. Many cases of acquired bone marrow failure do not fit cleanly into established MDS categories, yet an alternative diagnosis is not apparent. The term “idiopathic cytopenias of undetermined significance” (ICUS) has been proposed to describe these cases, but there is a paucity of information about the natural history of ICUS. New data on the natural history of MDS associated with a broad range of cytogenetic abnormalities that were not included in the International Prognostic Scoring System (IPSS), as well as the emerging picture of karyotypically occult DNA changes, promise to inform future classifications.

Haematopathology of classic myeloproliferative neoplasms

2020 ◽  
pp. 45-62
Author(s):  
Hans Michael Kvasnicka ◽  
Jürgen Thiele
Keyword(s):  
Continuous Improvement ◽  
Who Classification ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
World Health ◽  
Polycythaemia Vera ◽  
The World ◽  
Health Organization ◽  
Diagnostic Importance

The classification of the World Health Organization (WHO) continues to advocate the diagnostic importance of bone marrow (BM) morphology in the diagnostic workup of myeloproliferative neoplasms (MPN). In this regard, distinctive histological BM patterns characterize specific subtypes of MPN and are the key to a meaningful clinical and molecular-defined risk stratification of patients. In this regard, the morphological denominator includes a characteristic megakaryocytic proliferation along with variable changes in the granulopoiesis and erythropoiesis. Importantly, diagnosis of MPN requires absence of relevant dysgranulopoiesis or dyserythropoiesis. In terms of clinical practice, the concept of precursor stages provides the possibility of an early intervention by appropriate therapeutic regimens that might prevent fatal complications like thrombosis and haemorrhage, especially in early stages of polycythaemia vera or in primary myelofibrosis. However, the WHO classification is not aimed to capture all biological true cases of MPN or guarantee a complete diagnostic specificity and thus might be in need of continuous improvement following clinical experience.

scholarly journals Congenital eruption cyst: a case report

2010 ◽  
Vol 21 (3) ◽  
pp. 259-262 ◽  
Author(s):  
Ramón Manuel Alemán Navas ◽  
María Guadalupe Martínez Mendoza ◽  
Mário Roberto Leonardo ◽  
Raquel Assed Bezerra da Silva ◽  
Henry W. Herrera ◽  
...  
Keyword(s):  
Case Report ◽  
Tooth Extraction ◽  
Who Classification ◽  
World Health ◽  
Close Monitoring ◽  
Cystic Lesions ◽  
Separate Entity ◽  
Epithelial Cysts ◽  
Health Organization

Congenital pathologies are those existing at or dating from birth. Occurrence of congenital cystic lesions in the oral cavity is uncommon in neonates. Eruption cyst (EC) is listed among these unusual lesions. It occurs within the mucosa overlying teeth that are about to erupt and, according to the current World Health Organization (WHO) classification of epithelial cysts of the jaws, EC is a separate entity. This paper presents a case of congenital EC successfully managed by close monitoring of the lesion, without any surgical procedure or tooth extraction. Eruption of the teeth involved, primary central incisors, occurred at the fourth month of age. During this time neither the child nor mother had any complication such as pain on sucking, refusal to feed, airway obstruction, or aspiration of fluids or teeth.

Expert Second Opinion Pathology Review of Lymphoma in the Era of the World Health Organization Classification.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3317-3317
Author(s):  
Matthew J. Matasar ◽  
Weiji Shi ◽  
Jonathan Silberstien ◽  
Julie T. Feldstein ◽  
Daniel Filippa ◽  
...  
Keyword(s):  
World Health Organization ◽  
Who Classification ◽  
Second Opinion ◽  
World Health ◽  
Cancer Center ◽  
Major Revision ◽  
The World ◽  
Health Organization ◽  
Pathology Review

Abstract Background: The effective management of lymphoma depends upon an accurate and precise pathologic diagnosis. However, the classification of lymphoma continues to evolve. Reports addressing the role of second opinion expert pathology review have found varying impact, and little is known regarding the predictors of a change in diagnosis. Furthermore, the impact of the World Health Organization (WHO) classification of lymphomas over the 5 years following their formal publication has not been formally assessed. Methods: All outside pathology is reviewed at Memorial Sloan-Kettering Cancer Center (MSKCC) before a clinical opinion is finalized. We performed a chart review of all externally referred lymphoma cases from 1/1/01 to 6/30/01 and from 1/1/06 to 6/30/06 with second opinions from MSKCC hematopathology. Statistical analysis was performed using Chi-square or Fisher’s exact test for univariate analysis and logistic regression for multivariate analysis. Results: 719 patients (365 in 2001, 354 in 2006) met inclusion criteria. Diagnostic revisions were classified as major or minor; major changes were those that would lead to management changes as per National Comprehensive Cancer Network guidelines. 122 patients (18% in 2001, 16% in 2006) had a major diagnostic revision and an additional 22 (4% in 2001, 2% in 2006) had confirmation of major revisions rendered previously at second opinion from another National Cancer Institute Comprehensive Cancer Center (CCC). This did not change significantly by era, with 79 major revisions (22%) in 2001 and 65 (18%) in 2006 (P=NS). An additional 55 patients [24 (7%) in 2001, 31 (9%) in 2006] received minor revisions. Common categories of major revision included changing from nondiagnostic/ambiguous to definitive [6 in 2001, 8 in 2006], definitive to nondiagnostic [9 in 2001, 9 in 2006], malignant to benign [1 in 2001, 6 in 2006], indolent B-cell lymphoma (BCL) to aggressive BCL [15 in 2001, 8 in 2006], and aggressive BCL to indolent BCL [4 in 2001, 1 in 2006]. Major diagnostic revision was significantly associated with additional immunohistochemistry (IHC) testing in 2001 (OR=2.3; 95%CI 1.3, 4). In 2006, additional IHC (OR=1.8; 95%CI 1, 3.4), repeat biopsy (OR=3.1; 95%CI 1.2, 8.0), and skin biopsy (versus lymph node biopsy; OR 3.3; 95%CI 1.6, 7.0) were significantly associated with major revision. Two of the 7 patients reclassified as benign received revisions based on additional IHC, whereas 7 of the 14 patients reclassified as malignant were revised due to either additional IHC (4) or repeat biopsy (3). No effect was seen by biopsy type, nor were patient gender, age, race or ethnicity associated with odds of major revision. Of cases seen first at another CCC, 12% in 2001 and 16% in 2006 received major revisions, compared to 19% (2001) and 16% (2006) of other cases; these differences were not statistically significant. Conclusion: The rate of clinically meaningful diagnostic revisions at second opinion expert pathology review was high for patients seen at MSKCC, and remained so despite five years of increased familiarity with the WHO classification schema. These data confirm the fact that an appropriate evaluation, including detailed IHC and an adequate biopsy specimen, plays a central role in the accurate diagnosis of lymphoma. The high rates of diagnostic revision reported here lend support to the routine application of expert second opinion hematopathology review.

Retrospective Analysis of Patients with Refractory Cytopenia with Unilineage Dysplasia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4847-4847
Author(s):  
Yuchun Wang ◽  
Xin Du ◽  
Suxia Geng
Keyword(s):  
Myelodysplastic Syndromes ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Major Change ◽  
Classification Systems ◽  
World Health ◽  
Refractory Anemia ◽  
Prognostic Information ◽  
Prognostic Assessment ◽  
Health Organization

Abstract Abstract 4847 Background Myelodysplastic syndromes (MDS) are a group of clonal heterogeneous bone marrow stem cell disorders characterized by dysplastic hypercellular marrows with peripheral cytopenias. Accurate diagnosis and classification are essential for subgroup identification and prognostic assessment of patients with MDS. Classification systems such as the World Health Organization (WHO) classification are widely used but do not always provide sufficient prognostic information. So WHO improved the classification systems last year(2008). The major change was that refractory anemia (RA) was substituted by refractory cytopenia with unilineage dysplasia (RCUD). The aim of this study was to apply the new WHO classification to re-evaluate MDS with RA. Methods MDS patients with RA diagnosed between 2000 and 2008 were retrospectively reclassified with WHO criteria (2008). Results According to the new WHO classification, RCUD is intended to encompass those myelodysplastic syndromes (MDS) which present with a refractory cytopenia with unilineage dysplasia and includes refractory anemia (RA), refractory neutropenia (RN) and refractory thrombocytopenia (RT), so in 34 cases diagnosed as RA, 12 cases (12/34,35%) still pertained to RA, 19 cases (56% ) to RN and 3 (9%) cases to RT. IPSS showed 94% of RCUD cases categorized as low or intermediate-I risk. The median survival of patients with RCUD was 84 months. Conclusions RA is the main subtypes of RCUD. Understanding the clinical features of RCUD accurately is helpful to determine the right subtype of RCUD. The new WHO classification system was improved to provide better diagnostic criteria. Disclosures No relevant conflicts of interest to declare.

scholarly journals Intracranial Gliofibroma: A Case Report and Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Patricia Gargano ◽  
Graciela Zuccaro ◽  
Fabiana Lubieniecki
Keyword(s):  
Who Classification ◽  
Clinical Symptoms ◽  
Mass Effect ◽  
World Health ◽  
Biological Behavior ◽  
Positive Staining ◽  
Immunohistochemical Examination ◽  
Collagenous Stroma ◽  
Health Organization

Gliofibroma is a rare tumor with biphasic morphology, commonly occurring in the first two decades of life. Currently, the tumor is not listed as a distinct entity in the current World Health Organization (WHO) classification of central nervous system tumors. As its biological behavior, histogenesis, and prognostic factors are still debated, the aim of this paper was to describe a case of a gliofibroma and to update the data about these lesions. Hence, we present here clinical symptoms, pathological findings, and evolution observed in a child with gliofibroma. A 10-year-old girl with seizures was referred for study. Neuroimaging showed a hemispheric hyperdense tumor with little peritumoral edema and no mass effect. The tumor was totally removed. Histologically, the tumor consisted of a mixture of glial cells and collagen-rich stroma. Immunohistochemical examination revealed positive staining for GFAP, CD 99, S100, and vimentin. EMA staining showed a paranuclear dot pattern in only few cells in isolated areas. These findings of a glial component with collagenous stroma were consistent with a desmoplastic glioma. Because of the rarity of this entity, we believe it is important to report every case in order to adequately analyze and categorize the tumor in the next WHO classification.

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